Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027819 (neuroblastoma)
 27,800 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A case of meningeal carcinomatosis associated with cerebral metastases from an adrenal neuroblastoma is described. The clinical picture was ushered-in by bilateral sciatic pain in a 50 years old female and was followed by rapidly progressive sensory-motor deficits of the arms and legs, leading to flaccid quadriplegia associated with paralysis of cranial nerves and episodes of mental confusion. Death occurred 4 months alter, in cardiac failure. At autopsy, a bilateral tumor of the adrenal glands was found. No metastases were detected anywhere except in the central nervous system. Histology identified the tumor as a neuroblastoma; meningeal carcinomatosis, radicular infiltration by tumor cells and parenchimal metastases were found in the central nervous system. Neuroblastoma is typically a tumor of childhood, only 13% of them being found in adult's according to Russell and Rubinstein. Meningeal metastases from adrenal neuroblastoma have not hitherto been reported in the literature. In our opinion, the most likely mode of spread of tumor cells to the central nervous system was hematogenous because of the presence of small multiple intraparenchimal metastases; however, possible spread through the perineural lymphatics, as proposed by others, cannot be excluded, due to the prominent localization of tumor cells at spinal roots level. The main differential diagnostic problems (paraneoplastic neuropathy (Wyburn-Mason) and infectious subacute or chronic meningitis) are discussed. The authors stress the emportance of complete cerebro-spinal fluid examination including a careful search for tumor cells.
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PMID:[Meningeal carcinomatosis: clinical and anatomical study of a case of suprarenal neuroblastoma (author's transl)]. 6

In children with primary extracranial neuroblastoma (NB), intrinsic central nervous system (CNS) metastases (brain parenchyma or leptomeninges) are thought to occur rarely. This study was done to evaluate our anecdotal experience, which suggested that CNS involvement is becoming more frequent. Reports of computed tomographic (CT) and magnetic resonance (MR) imaging scans, biopsies, cerebrospinal fluid (CSF) cytologies, and autopsies were reviewed for children with stage IV NB diagnosed in 1978-1993 and followed at the Children's Hospital of Pittsburgh. Of 43 children over the age of 1 year, CNS metastases were documented in 7 (16.2%). Six patients developed signs or symptoms best explained by the presence of CNS tumor and had radiographic and/or histologic evidence of parenchymal disease (cortical masses on CT and MR, n = 3; suprasellar mass on CT, n = 1; diffuse leptomeningeal carcinomatosis by MR and/or autopsy, n = 2). CSF cytologies were positive in the one patient so tested. An additional asymptomatic patient had extensive CNS involvement at autopsy. In two of these children, the CNS was the first or only site of recurrent disease. It is concluded that intrinsic CNS disease is not uncommon in children with NB over the age of 1 year and there has been a trend toward its increasing recognition in recent years. Whether this is a function of wider use of diagnostic tools or a true change in natural history over time with increased intensity of chemotherapy is not clear. A study that prospectively monitors children with advanced neuroblastoma, radiographically and with CSF cytologies (prior to treatment and at 6-monthly intervals), is under way and should help to better define the natural history in the context of current therapies.
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PMID:Recognition of central nervous system metastases in children with metastatic primary extracranial neuroblastoma. 918 8

Labeling of specific antibodies with bifunctional chelated Actinium-225 ((225)Ac; an alpha generator) allows the formation of new, highly potent and selective alpha-emitting anticancer drugs. We synthesized and evaluated a radioimmunoconjugate based on 3F8, an IgG(3) antibody that specifically binds to ganglioside GD2, which is overexpressed by many neuroectodermal tumors including neuroblastoma. The (225)Ac-1,4,7,10-tetra-azacylododecane (DOTA)-3F8 construct was evaluated for radiochemical purity and sterility, immunoreactivity, cytotoxicity in vitro, induction of apoptosis on GD2-positive cells, as well as for pharmacological biodistribution and metabolism of the (225)Ac generator and its daughters in a nude mouse xenograft model of neuroblastoma. The (225)Ac-3F8 showed an IC(50) of 3 Bq/ml (80 pCi/ml) on the neuroblastoma cell line, NMB7, in vitro. Apoptosis of these cells was not observed. Biodistribution in mice showed specific targeting of a subcutaneous tumor; there was redistribution of the (225)Ac daughter nuclides mainly from blood to kidneys and to small intestine. Toxicity was examined in cynomolgus monkeys. Monkeys injected with 1 to 3 doses of intrathecal (225)Ac-3F8 radioimmunoconjugate (80 to 150 kBq/kg total dose) did not show signs of toxicity based on blood chemistry, complete blood counts, or by clinical evaluations. Therapeutic efficacy of intrathecal (225)Ac-3F8 was studied in a nude rat xenograft model of meningeal carcinomatosis. The (225)Ac-3F8 treatment improved survival 2-fold from 16 to 34 days (P = 0.01). In conclusion, in vivo alpha generators targeted by 3F8 warrant additional study as a possible new approach to the treatment of carcinomatous meningitis.
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PMID:Treatment of neuroblastoma meningeal carcinomatosis with intrathecal application of alpha-emitting atomic nanogenerators targeting disialo-ganglioside GD2. 1550 78

During over 50 years of the first author's career in neuropathology at Montefiore Medical Center in New York, we have come across certain interesting neuropathological findings. In this communication, some photographs showing macroscopic, microscopic and electron microscopic significant findings are selected to illustrate usefulness not only for the diagnosis but also for understanding of the nervous system. The six topics presented in this paper are: (i) unattached presynaptic terminals in cerebellar neuroblastoma; (ii) neurofibrillary tangle formation in the nucleus basalis of Meynert ipsilateral to a massive cerebral infarct; (iii) orderly arrangement of tumor cells in leptomeningeal carcinomatosis; (iv) interface between craniopharyngioma and brain tissue; (v) neurofibrillary tangles and Lewy bodies in a single neuron; and (vi) Cu/Zn superoxide dismutase positive Lewy body-like hyaline inclusions in anterior horn cells in familial motor neuron diseases. Analyses of these findings are presented for an educational purpose.
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PMID:Diagnostic clues and more from photographs. 1731 78

During over 50 years of my career in Neuropathology at Montefiore Medical Center in New York, I have come across certain interesting neuropathological findings. In this communication, some photographs showing macroscopic, microscopic and electron microscopic significant findings are selected to illustrate the usefulness, not only for the diagnosis but also for the understanding of the nervous system. The 11 topics presented in this paper are: (1) alteration of dura mater associated with advanced aging; (2) orderly arrangement of tumor cells in leptomeningeal carcinomatosis; (3) horizontal section of brain with border zone infarct; (4) neurofibrillary tangle formation in the nucleus basalis Meynert ipsilateral to a massive cerebral infarct; (5) extracellular spread of hematogenous edema fluid in the white matter: (6) unrolled myelin sheath: (7) unattached presynaptic terminals in cerebellar neuroblastoma: (8) unattached post synaptic terminals in agranular cerebellar degeneration: (9) neurofibrillary tangles and Lewy bodes in a single neuron: (10) Cu/Zu superoxide dismutase positive Lewy body-like hyaline inclusions in anterior horn cells in familial motor neuron disease: (11) Hirano body. Analysis of these findings are presented for an educational purpose.
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PMID:[Neuropathological perspective on single slide]. 1919 88

Disseminated carcinomatosis of the bone marrow (DCBM) is a condition in which bone marrow (BM) metastases diffusely invade the BM, and is frequently accompanied by disseminated intravascular coagulation (DIC). While prostate, lung, breast and stomach malignancies, in addition to neuroblastoma, are the most prevalent non-hematological malignancies to metastasize frequently to the BM, colorectal cancer is a malignancy that rarely metastasizes to the BM. The present case describes a 65-year-old male patient treated by resection and one course adjuvant chemotherapy for stage IIIC rectal cancer who presented with nasal bleeding at 8 months post-surgery. A blood test exhibited DIC. A BM biopsy was performed and the definitive diagnosis was DCBM with DIC. Promptly, anti-DIC treatment and chemotherapy with a modified FOLFOX6 (folinic acid, leucovorin (LV), 5-fluorouracil (5-FU) and oxaplatin) regimen was started. Following 1 cycle of chemotherapy, DIC was improved and subsequent to 2 cycles of modified FOLFOX6 the patient was discharged. The patient was alive 263 days subsequent to the diagnosis of DIC, but succumbed to carcinomatous meningitis as a result of disease progression. To the best of our knowledge, this is the first report of DCBM with DIC of curatively resected rectal cancer as the first presentation of relapse that was successfully treated with aggressive therapy, including chemotherapy.
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PMID:Disseminated carcinomatosis of the bone marrow with disseminated intravascular coagulation as the first symptom of recurrent rectal cancer successfully treated with chemotherapy: A case report and review of the literature. 2859 29